Difference between revisions of "Eosinophilic granulomatosis with polyangiitis"
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(similar name to what was prev Wegener's) |
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| Width = | | Width = | ||
| Caption = Eosinophilic granulomatosis with polyangiitis. [[H&E stain]]. | | Caption = Eosinophilic granulomatosis with polyangiitis. [[H&E stain]]. | ||
| Synonyms = | | Synonyms = Churg-Strauss syndrome, Churg-Strauss disease | ||
| Micro = small vessel [[vasculitis]] with abundant eosinophils and [[granuloma]]s | | Micro = small vessel [[vasculitis]] with abundant eosinophils and [[granuloma]]s | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = | | LMDDx = eosinophilic vasculitis associated with a [[connective tissue disease]] | ||
| Stains = | | Stains = | ||
| IHC = | | IHC = | ||
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| Symptoms = | | Symptoms = | ||
| Prevalence = rare | | Prevalence = rare | ||
| Bloodwork = | | Bloodwork = ANCA +ve/-ve | ||
| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
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| Tx = | | Tx = | ||
}} | }} | ||
'''Eosinophilic granulomatosis with polyangiitis''', previously known as '''Churg-Strauss syndrome''' | '''Eosinophilic granulomatosis with polyangiitis''', abbreviated '''EGPA''',<ref>{{Cite journal | last1 = Xu | first1 = XL. | last2 = Song | first2 = W. | last3 = Sui | first3 = X. | last4 = Song | first4 = L. | last5 = DU | first5 = QN. | last6 = Wang | first6 = X. | title = Radiological and Clinical Features of Eosinophilic Granulomatosis with Polyangiitis. | journal = Zhongguo Yi Xue Ke Xue Yuan Xue Bao | volume = 38 | issue = 5 | pages = 617-620 | month = Oct | year = 2016 | doi = 10.3881/j.issn.1000-503X.2016.05.023 | PMID = 27825424 }}</ref> is a type of [[vasculitis]]. | ||
It was previously known as '''Churg-Strauss syndrome'''.<ref name=pmid26051917>{{Cite journal | last1 = Stone | first1 = JR. | last2 = Bruneval | first2 = P. | last3 = Angelini | first3 = A. | last4 = Bartoloni | first4 = G. | last5 = Basso | first5 = C. | last6 = Batoroeva | first6 = L. | last7 = Buja | first7 = LM. | last8 = Butany | first8 = J. | last9 = d'Amati | first9 = G. | title = Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases. | journal = Cardiovasc Pathol | volume = 24 | issue = 5 | pages = 267-78 | month = | year = | doi = 10.1016/j.carpath.2015.05.001 | PMID = 26051917 }}</ref> | |||
It should '''''not''''' be confused with ''[[granulomatosis with polyangiitis]]'', previously known as ''Wegener's granulomatosis''. | |||
==General== | ==General== | ||
| Line 39: | Line 43: | ||
*'''F'''ever. | *'''F'''ever. | ||
*'''E'''osinophilia. | *'''E'''osinophilia. | ||
Other clinical features - may be present:<ref name=pmid27671089 >{{Cite journal | last1 = Cottin | first1 = V. | last2 = Bel | first2 = E. | last3 = Bottero | first3 = P. | last4 = Dalhoff | first4 = K. | last5 = Humbert | first5 = M. | last6 = Lazor | first6 = R. | last7 = Sinico | first7 = RA. | last8 = Sivasothy | first8 = P. | last9 = Wechsler | first9 = ME. | title = Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). | journal = Autoimmun Rev | volume = | issue = | pages = | month = Sep | year = 2016 | doi = 10.1016/j.autrev.2016.09.018 | PMID = 27671089 }}</ref> | |||
*ANCA. | |||
*Weight loss. | |||
*Myalgias. | |||
*Arthralgias | |||
*Glomerulonephritis. | |||
*Hematuria. | |||
Notes: | Notes: | ||
*Similar to [[Granulomatosis with polyangiitis]] (Wegener's granulomatosis) - classically c-ANCA +ve, and ''microscopic polyangiitis''.<ref>URL: [http://emedicine.medscape.com/article/334024-overview http://emedicine.medscape.com/article/334024-overview]. Accessed on: 22 January 2011.</ref> | *Similar to [[Granulomatosis with polyangiitis]] (Wegener's granulomatosis) - classically c-ANCA +ve, and ''microscopic polyangiitis''.<ref>URL: [http://emedicine.medscape.com/article/334024-overview http://emedicine.medscape.com/article/334024-overview]. Accessed on: 22 January 2011.</ref> | ||
*T<sub>H</sub>2 disease - like [[asthma]].<ref name=pmid25500434>{{Cite journal | last1 = Greco | first1 = A. | last2 = Rizzo | first2 = MI. | last3 = De Virgilio | first3 = A. | last4 = Gallo | first4 = A. | last5 = Fusconi | first5 = M. | last6 = Ruoppolo | first6 = G. | last7 = Altissimi | first7 = G. | last8 = De Vincentiis | first8 = M. | title = Churg-Strauss syndrome. | journal = Autoimmun Rev | volume = 14 | issue = 4 | pages = 341-8 | month = Apr | year = 2015 | doi = 10.1016/j.autrev.2014.12.004 | PMID = 25500434 }}</ref> | |||
==Microscopic== | ==Microscopic== | ||
Latest revision as of 21:50, 28 November 2016
| Eosinophilic granulomatosis with polyangiitis | |
|---|---|
| Diagnosis in short | |
 Eosinophilic granulomatosis with polyangiitis. H&E stain. | |
|
| |
| Synonyms | Churg-Strauss syndrome, Churg-Strauss disease |
|
| |
| LM | small vessel vasculitis with abundant eosinophils and granulomas |
| LM DDx | eosinophilic vasculitis associated with a connective tissue disease |
| Site | blood vessels |
|
| |
| Associated Dx | asthma |
| Signs | fever |
| Prevalence | rare |
| Blood work | ANCA +ve/-ve |
Eosinophilic granulomatosis with polyangiitis, abbreviated EGPA,[1] is a type of vasculitis.
It was previously known as Churg-Strauss syndrome.[2]
It should not be confused with granulomatosis with polyangiitis, previously known as Wegener's granulomatosis.
General
Defining features - memory device GAFE:
- Granulomata.
- Asthma.
- Fever.
- Eosinophilia.
Other clinical features - may be present:[3]
- ANCA.
- Weight loss.
- Myalgias.
- Arthralgias
- Glomerulonephritis.
- Hematuria.
Notes:
- Similar to Granulomatosis with polyangiitis (Wegener's granulomatosis) - classically c-ANCA +ve, and microscopic polyangiitis.[4]
- TH2 disease - like asthma.[5]
Microscopic
Features:
- Small vessel vasculitis.
- Abundant eosinophils.
- Granulomas.
DDx:
- Eosinophilic vasculitis associated with a connective tissue disease.[6]
Images
CSS - high mag. (WC)
CSS - very high mag. (WC)
See also
References
- ↑ Xu, XL.; Song, W.; Sui, X.; Song, L.; DU, QN.; Wang, X. (Oct 2016). "Radiological and Clinical Features of Eosinophilic Granulomatosis with Polyangiitis.". Zhongguo Yi Xue Ke Xue Yuan Xue Bao 38 (5): 617-620. doi:10.3881/j.issn.1000-503X.2016.05.023. PMID 27825424.
- ↑ Stone, JR.; Bruneval, P.; Angelini, A.; Bartoloni, G.; Basso, C.; Batoroeva, L.; Buja, LM.; Butany, J. et al. "Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases.". Cardiovasc Pathol 24 (5): 267-78. doi:10.1016/j.carpath.2015.05.001. PMID 26051917.
- ↑ Cottin, V.; Bel, E.; Bottero, P.; Dalhoff, K.; Humbert, M.; Lazor, R.; Sinico, RA.; Sivasothy, P. et al. (Sep 2016). "Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss).". Autoimmun Rev. doi:10.1016/j.autrev.2016.09.018. PMID 27671089.
- ↑ URL: http://emedicine.medscape.com/article/334024-overview. Accessed on: 22 January 2011.
- ↑ Greco, A.; Rizzo, MI.; De Virgilio, A.; Gallo, A.; Fusconi, M.; Ruoppolo, G.; Altissimi, G.; De Vincentiis, M. (Apr 2015). "Churg-Strauss syndrome.". Autoimmun Rev 14 (4): 341-8. doi:10.1016/j.autrev.2014.12.004. PMID 25500434.
- ↑ Chen, KR.; Su, WP.; Pittelkow, MR.; Conn, DL.; George, T.; Leiferman, KM. (Aug 1996). "Eosinophilic vasculitis in connective tissue disease.". J Am Acad Dermatol 35 (2 Pt 1): 173-82. PMID 8708015.